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Hale ED, 12. Repke J, Neill J, et al. Lupus. Linking … / ABSTRACT: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease most commonly involving skin, joints, and kidneys. FDA news release. Alarcón GS, Overall, 50 percent of patients with systemic lupus erythematosus have some manifestation of lupus nephritis; this figure reached 75 percent in black patients who were followed prospectively by the author. Rheinschmidt M, 1. Steinberg AD, Klippel JH, Treatment algorithms in systemic lupus erythematosus. Satoh M, Marchand-Adam S, 10. Dall'era M, Wofsy D. Clinical features of systemic lupus erythematosus. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Lassila O. Diot E. In addition, NSAIDs may adversely affect renal function, a special concern because 50 percent of patients with systemic lupus erythematosus develop associated nephritis. Fessel WJ. Clin Immunol Immunopathol. The 1982 revised criteria for the classification of systemic lupus erythematosus. 2003;82(5):299–308. Carter BL, 7. Information from references 8 through 11, and 18 through 21. Merrill JT, The 1982 revised criteria for the classification of systemic lupus erythematosus. Tench CM, Antinuclear, anticytoplasmic, and anti-Sjogren's syndrome antigen A (SS-A/Ro) antibodies in female blood donors. 1996;39:1791–801. et al. Patients with mild SLE do not normally require use of systemic corticosteroids but there are patients whose quality of life is not improved if not given low-dose corticosteroids. Systemic lupus erythematosus is a disease that continues to evolve over time. 40. Hydroxychloroquine is very safe; only 1 percent of patients using it develop retinopathy. Hughes GR. / afp Osteonecrosis, also called avascular necrosis of bone, occurred in 14 percent of patients in one study.8, Osteonecrosis (avascular necrosis of bone), Occurs in 14 percent of patients with systemic lupus erythematosus, Core decompression of bone is an effective treatment in early stages of the disease, Occurs in 64 percent of patients with systemic lupus erythematosus, Osteoporosis of the lumbar spine is associated with the highest dosage of prednisone and the cumulative effects of prednisone, Calcium, vitamin D, calcitonin and bisphosphonates are effective treatments (even in premenopausal women with osteoporosis). paucity of evidence in areas such as biomarkers, signs and symptoms for the monitoring and . Immediate, unlimited access to all AFP content. 9th ed. 1982;25:1271–7. SLE is an independent risk factor for cardiovascular disease (CVD), due to both traditional and disease-related risk factors, such as persistent disease activity, LN, presence of aPL and use of … Katzap E. Severe hemolytic anemia is treated initially with intravenous methylprednisolone, 1,000 mg per day for three days. Cervera R, Bijlsma JW, Spence D, Continued monitoring, even when the disease appears to be clinically inactive, is essential. J Rheumatol. National Arthritis Data Workgroup. Goldberg A, Data Sources: Searches were done in Essential Evidence Plus, Clinical Evidence, the Cochrane Database of Systematic Reviews, and DynaMed. Some patients with very severe cases of discoid lupus erythematosus may not respond adequately to the usual dosage of hydroxychloroquine, which is 400 mg per day for a normal-sized adult. Richards HB, 2000;55(2):159–166. Selton-Suty C, Scopelitis E. Systemic lupus erythematosis. 2003;68(11):2179–2187. European Working Party on Systemic Lupus Erythematosus. Alarcón GS. Fries JF, Orbai AM, This requires compliance with office visits and medications, and lifestyle modifications to reduce or prevent associated problems such as hyperlipidemia, obesity and hypertension. Hallet DC. Am J Epidemiol. Petri M, Ocular manifestations of systemic lupus erythematosus. Balsamo S, The aim of this guideline was to produce recommendations for the management of adult lupus patients in the UK that cover the diagnosis, assessment and monitoring of lupus and the … Classic autoimmune hemolytic anemia can present acutely (and severely) or as a chronic condition. Circulation. 2018 Jan 1;57(1):e1-e45. et al. 2002;41(10):1095–1100. Joseph L, Management of SLE depends on the level of disease activity and can include general measures, NSAIDs and steroids. Yazdany J, Mujic F, Wilkinson A, The management of thrombosis in the antiphospholipid-antibody syndrome. Font J, Curr Cardiol Rev. Because systemic lupus erythematosus is a chronic disease, patients require extensive health education in terms of their responsibility in managing their condition. Derksen RH. Branch DW, 4. LUpus in MInority populations: NAture vs Nurture. One of the most effective drugs in the treatment of cutaneous lupus erythematosus, including discoid lupus erythematosus, is thalidomide (Synovir); however, because of its teratogenic effects and the increased risk of peripheral neuropathy in patients taking it, this agent will probably never have widespread use. Bae SC, 2012;61(40):816–819. 2006;65(5):585–589. Ruiz-Irastorza G, Trujillo-Martín MM, Patients with SLE should use sunscreens with sun protective factors of 15 or higher.32 Mucous membrane involvement, hair loss, and Raynaud phenomenon are also common (Figure 4). Bruce IN, 10. Choose a single article, issue, or full-access subscription. This review updates a previous article on this topic by Gill, et al. This clinical content conforms to AAFP criteria for continuing medical education (CME). Lynch JP III. Recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Balow JE. Sinicato NA, Balow JE. Supplementation with folic acid during methotrexate therapy for rheumatoid arthritis. Bernatsky S, Predictors of organ damage in systemic lupus erythematosus: the Hopkins Lupus Cohort. Nadeau MR, Yazdany J, Systemic lupus erythematosus (SLE) is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues. Usually, it presents in women in puberty or early adulthood. 25. Petri M, Ioannidis JP, Systemic lupus erythematosus in the community. Information from references 8, 24 through 27, 55, and 56. Patients diagnosed with SLE must meet at least four of the 11 American College of Rheumatology diagnostic criteria. Thorax. The natural history of hypercholesterolaemia in systemic lupus erythematosus. One of the underlying defects in systemic lupus erythematosus may center on apoptosis, or programmed cell death. Systemic lupus erythematosus (SLE) is a challenging disorder for clini- cians and those affected. Petri M, 2007;46(12):1757–1762. Mujic F, Hartkamp A, http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm246489.htm. Clinical significance of antinuclear antibodies in systemic rheumatic diseases. doi: 10.1093/rheumatology/kex286. The Hopkins Lupus Pregnancy Center: 1987–1991 update. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. 33. This partnership has been summarized in the guidelines from the ACR.6. Purvey S, 2010;69(1):20–28. In press. Austin JS, In patients who cannot tolerate antimalarials, dapsone or retinoids are additional therapeutic choices. Marder W, Magder LS. Meilahn EN, Roubenoff R, et al. 1997;145(5):408–415. 53. Aringer M, Incidence, prevalence, outcome, and first symptoms; the high prevalence in black women. SLE is a multi-system autoimmune disease that is characterised by mostly chronic inflammatory effects on a variety of organs. Silosi I, Guidelines for referral and management of systemic lupus erythematosus in adults. Morgan SL, Lassila O. 15. Twenty years ago, women with systemic lupus erythematosus were often told not to become pregnant. Lee JV, Gordon C. McCurdie I, Tonner C, McShane DJ, The major risk factors for osteonecrosis include a prednisone dosage greater than 20 mg per day for one month or longer, and the presence of Raynaud's disease or vasculitis.8 Avascular necrosis of bone, if detected early by magnetic resonance imaging (MRI), can often be effectively treated with core decompression of bone, an orthopedic procedure.9 Patients with osteonecrosis who present at later stages usually require a total joint replacement. Helmick CG, Curtis KM, Anteriorly, they are: low cervical, second rib, lateral epicondyle, knee, Arthralgia, fever, lymphadenopathy, malaise, myalgia, peripheral neuropathy, rash, Western blot assay for detection of HIV antibodies, Diarrhea, peripheral arthritis, rectal bleeding, tenesmus, Colonoscopy to assess disease activity; measure CRP level, platelets, and ESR; test for anemia, Arthritis, carditis, erythema migrans, neuritis, Arthralgia, myalgia, puffy fingers, Raynaud phenomenon, sclerodactyly, Tests for elevated ESR and hypergammaglobulinemia, positive anti-U1RNP antibodies, Psoriasis before joint disease, nail changes in fingers and toes, Inflammatory articular disease and more than three of the following: psoriasis, nail changes, negative rheumatoid factor, dactylitis, radiographic evidence of new bone formation in hand or foot, Acute nonpurulent arthritis from infection elsewhere in the body, Clinical diagnosis to identify triggers; serologic findings of recent infections may be present, Morning joint stiffness lasting more than one hour; affected joints are usually symmetric, tender, and swollen, Positive tests for rheumatoid factor and anticyclic citrullinated antibodies; synovial fluid reflects inflammatory state, Cough, dyspnea, fatigue, fever, night sweats, rash, uveitis, Chest radiography, bilateral adenopathy with biopsy revealing non-caseating granuloma, elevated angiotensin-converting enzyme level, Arthralgia, decreased joint mobility, myalgia, Raynaud phenomenon, skin induration, Dry skin, fatigue, feeling cold, weakness. Harrison's Principles of Internal Medicine. Urowitz MB, Rheumatology (Oxford). da Silva Cardoso PA, Hale ED, Increased risk for heart valve disease associated with antiphospholipid antibodies in patients with systemic lupus erythematosus: meta-analysis of echocardiographic studies. FDA news release. ), In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) revised and validated the ACR criteria.13 In the SLICC system, a patient must meet at least four criteria, including at least one abnormal clinical criterion and one abnormal immunologic criterion, or the patient must have biopsy-confirmed lupus nephritis and elevated ANA or anti-dsDNA levels.13 The SLICC criteria, which have not been endorsed by the ACR, have greater sensitivity (97%) but lower specificity (84%) compared with the ACR criteria.13 Because the SLICC criteria have not been tested for the purpose of diagnosis, the ACR criteria continue to be the diagnostic standard.13, Table 2 compares the ACR and SLICC criteria.8,13, There are many manifestations of SLE, and multiple organ systems may be affected (Table 3).8–11,18–21 Fatigue and arthralgia are present in almost all patients with SLE, whereas renal involvement manifests in about 50% of patients.22 The most important predictors of disease progression are older age at diagnosis, black race, and low income.23, The management of SLE depends on the system involved. Robinson JG, Weisman MH, Mosca L, Petri M, Accessed April 1, 2015. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Ciurea PL. et al. McCarthy J, Cojocaru IM, Arch Intern Med. Petri M, Bijl M, Long-term complications of systemic lupus erythematosus. Howard D, Corash L. Klippel JH, Effectiveness-based guidelines for the prevention of cardiovascular disease in women—2011 update: a guideline from the American Heart Association [published corrections appear in Circulation. 1999;42(9):1785–1796. Berra K, This study aims to compare the scope, quality, and consistency of clinical practice guidelines on the diagnosis, monitoring, and treatment of patients with SLE. Use of combination antimalarial therapy (hydroxychloroquine [Plaquenil] and quinacrine) or chloroquine (Aralen), which has more risk of retinopathy, is sometimes necessary, Use of corticosteroids may be necessary as part of initial therapy for severe discoid lupus or for lupus vasculitis; intradermal corticosteroids are helpful for individual discoid lesions, especially in the scalp, Methotrexate (Rheumatrex) or azathioprine (Imuran) is used as steroid-sparing drug, One of the most effective drugs for treatment of discoid lupus, but teratogenicity and neuropathy will limit its acceptance and use. et al. de Pascual-Medina AM, 5. These antibodies often fluctuate over time, as does disease activity in general. Antimalarial agents, especially hydroxychloroquine (Plaquenil), are frequently used in the treatment of polyarthritis. Ann Intern Med. The early detection of avascular necrosis of bone usually requires an MRI scan of the hip. Alarcón GS, 4(August 15, 2016) Cowchock FS, Petri M, Quinacrine, in a dosage of 100 mg per day, can be added without increasing the risk of retinopathy, or the patient can be switched to chloroquine HCl (Aralen), in a dosage of 250 mg per day. Gladman D, et al. Morgan SL, Ioannidis JP, Diagnosis of systemic lupus erythematosus … Systemic lupus erythematosus in three ethnic groups. 31. Long-term complications of systemic lupus erythematosus. Kelly's Textbook of Rheumatology. White PD, Barbulescu AL, Tonner C, Patients with severe or resistant symptoms may also require treatment with intravenous cyclophosphamide and/or plasmapheresis. Quisel AM, 21. Perez-Gutthann S, Branch DW, However, it also has low specificity, and may be positive in healthy patients. Brey RL. Systemic lupus erythematosus. Hallet DC. 1985;36:120–8. Progression of avascular necrosis (AVN) of the femoral head in systemic lupus erythematosus: long-term follow-up report. The American College of Rheumatology has 11 classification criteria for lupus. Reprints are not available from the author. Diagnostic accuracy for lupus and other systemic autoimmune diseases in the community setting. Population-based incidence and prevalence of systemic lupus erythematosus: the Michigan Lupus Epidemiology and Surveillance program. Madhok R, Low-dose glucocorticoids can be used to treat most manifestations of lupus. Regnault V, If a patient meets at least four criteria, lupus can be diagnosed with 95% specificity and 85% sensitivity. Louthrenoo W, 48. SLE can be fatal and significantly increases the risk of cardiovascular disease. Joseph L, Casciola-Rosen L, Mortality in systemic lupus erythematosus. Goldman D. “Joining the dots” for patients with systemic lupus erythematosus: personal perspectives of health care from a qualitative study. Hahn BH, 42. Vaughn WH, A combination of glucocorticoid and mycophenolate (Cellcept) or cyclophosphamide is effective in achieving remission in patients with SLE nephritis. Petri M, A combination of glucocorticoid plus immunosuppressant is more effective than glucocorticoids alone in preserving renal function in patients with SLE. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. 4. Musculoskeletal involvement on systemic lupus erythematosus. 47. Godaert GL, Safety of contraceptive method use among women with systemic lupus erythematosus: a systematic review. A combination of glucocorticoid plus immunosuppressant is more effective than glucocorticoids alone in preserving renal function in patients with SLE.32 Standard treatment includes mycophenolate (Cellcept) or cyclophosphamide plus a glucocorticoid.27–29,34, The nervous system is affected in about 12% to 23% of patients with SLE.11 Neuropsychiatric manifestations of SLE include headaches, seizures, cerebrovascular disease, psychosis, cranial neuropathy, and movement disorder.35 Magnetic resonance imaging of the brain should be considered in patients with headache or seizure because white matter lesions are common in neuropsychiatric SLE.35 Treatment includes glucocorticoids and cyclophosphamide.26,30,31, Ocular manifestations are common in SLE. Treatment for active systemic lupus erythematosus differs, depending on the organ systems involved and disease severity. Am Fam Physician. McShane DJ, Navarra SV, Grodzicky T, Plouffe L. Carter BL, Compared with an age-matched group in the Framingham cohort, women 35 to 44 years of age who had SLE had a greatly increased risk of myocardial infarction (rate ratio = 52; 95% confidence interval, 22 to 98).42 The increased risk of accelerated atherosclerosis suggests that there are other SLE-related factors, such as renal disease, cytokines, inflammatory mediators, antiphospholipid antibodies, oxidized low-density lipoprotein, and adverse effects of treatment, that cause accelerated cardiovascular disease.19,43 SLE is an independent risk factor for the development of atherosclerosis, and is identified as such by the American Heart Association.19,44, It is important to counsel patients to reduce traditional cardiovascular risk factors such as smoking and obesity, and to have routine screenings for diabetes mellitus, hypertension, and dyslipidemia. 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